Phaeochromocytoma in black South Africans - a 30-year audit.

OBJECTIVE: Phaeochromocytomas are catecholamine-secreting tumours, the majority of which arise from the adrenal medulla. Untreated, they are potentially lethal; early diagnosis and treatment offer a good chance of cure. They are rarely reported in blacks. The clinical presentation and outcome of phaeochromocytoma in a large cohort of black South Africans is reviewed. METHODS: Patients' records in a tertiary care university hospital were reviewed. Fifty-four black patients presenting with phaeochromocytoma between 1980 and 2009 were included. The clinical presenting features, tumour localisation and outcome were assessed. RESULTS: Fifty-four (41 female, 13 male; age range 8 - 57 years) patients were identified. Five (9%) had familial syndromes; 49 (91%) were deemed sporadic. All tumours were intra-abdominal: 34 (61%) were adrenal and 22 (39%) extra-adrenal in origin. The most common symptoms were headache (77%), palpitations (77%), and sweating (74%). All were hypertensive, almost equally divided between paroxysmal and sustained hypertension. Six (11%) presented in congestive cardiac failure including 2 with catecholamine-induced myocarditis. Two patients had features which simulated hypertrophic obstructive cardiomyopathy. Nine women presented in pregnancy: there was no maternal mortality; fetal mortality included 1 early neonatal death and 1 intrauterine death. There were 4 deaths: 1 from postoperative haemorrhage, 1 from multisystem crisis, 1 from metastatic medullary thyroid carcinoma, and 1 from catecholamine-induced myocarditis. CONCLUSION: Phaeochromocytoma is an important although rare tumour in blacks, with similar clinical presentations and complications to those in white patients. Timely diagnosis and appropriate treatment resulted in a favourable outcome in over 90% of patients in this study.

Long-term (20 years) outcome and mortality of Type 1 diabetic patients in Soweto, South Africa.

AIMS: To assess the long-term (20 years) mortality, with causes of death, in a cohort of Type 1 diabetic patients resident in Soweto, South Africa. METHODS: A cohort of Type 1 diabetic patients attending the Diabetic Clinic of Baragwanath Hospital, Soweto were studied in 1982. They were followed over the subsequent 20 years, the final investigation being in 2002. Numbers dying during the period were recorded, as well as year of death and cause. The complication status of survivors was also assessed. RESULTS: Of the original cohort of 88 Type 1 patients, 21 died during the follow-up period. There were 39 lost to follow-up, giving a crude 20 years' mortality of 43%. Kaplan-Meier analysis showed mortality hazard of 33%. Of those dying, most (9/21) were as a result of renal failure. Other causes were hypoglycaemia (6), ketoacidosis (2), infection (2) and undetermined (2). Of the survivors, comparing data at 0 and 20 years' follow-up, there was a significant increase in rates of retinopathy (P<0.02) and hypertension (P<0.005), but not of other complications. CONCLUSIONS: This is the first long-term outcome study of Type 1 diabetes in sub-Saharan Africa. Although the mortality was substantial, it is similar to equivalent studies of United States (US) Afro-Americans with Type 1 diabetes. The major cause of death was renal failure related to diabetic nephropathy, and reflects lack of adequate facilities for renal replacement therapy. Despite the deprivation, poverty, political upheaval and recent AIDS epidemic in Soweto, Type 1 diabetes carries a reasonable long-term prognosis, and survivors are generally free of debilitating complications.

Phaeochromocytoma by way of case reports.

Endocrine causes of hypertension are relatively rare, but their detection offers a real chance for cure. This is particularly true of phaeochromocytoma, a catecholamine-producing tumour derived from chromaffin tissue, which, if left undetected, is invariably fatal. The lethal nature of this tumour is dependent on two major characteristics: firstly, its ability to secrete catecholamines in excess, resulting in potentially catastrophic consequences; and, secondly, its malignant potential. Approximately 5-10% of these tumours are malignant, which, if metastasised, are generally refractory to treatment. Clearly, however, because only one in 1,000 hypertensives is likely to harbour a phaeochromocytoma, it is not cost-effective option to screen all hypertensives for this cause. Rather, a selective approach is preferred in which a high index of suspicion for the clinical characteristics of this tumour is used to guide the physician. The following two case reports derived from our records at Chris Hani Baragwanath Hospital will illustrate many issues related to diagnosis and management of this fascinating tumour.